Associations of Budd-Chiari Syndrome and Factors Predicting Need for TIPS and Liver Transplantation: Long-Term Single-Center Experience

Background: Budd-Chiari Syndrome (BCS) is a rare thrombotic disorder that involves obstruction of hepatic venous outflow. It can result in increased sinusoidal pressure and portal hypertension. Patients may develop cirrhosis and liver failure from chronic injury, along with other complications such as ascites and varices. BCS is associated with thrombophilia and hematological disorders such as myeloproliferative neoplasms (MPNs) and other auto-immune diseases. Patients typically receive long-term anticoagulation to decrease the risk of recurrence of thrombosis along with supportive measures such as trans-jugular intrahepatic portosystemic shunt (TIPS) and liver transplant. Methods: We performed a retrospective study to describe the patient population with BCS at UNMC. We evaluated 23 patient medical records and collected data on demographics, risk factors, health history, BCS presentation, associated diseases, any acquired/inherited thrombophilia, laboratory results at the earliest available date from diagnosis, and treatments/procedures received. Model of End-Stage Liver Disease (MELD) and Child-Pugh scores were calculated and reported. Descriptive Findings: The median age for the 23 patients in our study was 36 years (range: 11-59 years). The percentage of females was 60.87%. The overall survival rate was 91.3%. Median follow-up duration was 89 months (range: 3-330 months). Of the total, 57% of the patients had myeloproliferative neoplasms (MPNs). Cirrhosis of liver was observed in 74% of the patients. 8.7% of patients developed BCS after receiving the COVID-19 vaccine (Ad26.COV2.S). 91.3% of patients received long-term anticoagulation. 65% of patients received TIPS and/or liver transplant. Conclusion: The most common primary cause of BCS in our study was MPNs (57%). Vaccine-induced thrombotic thrombocytopenia (VITT) after COVID-19 vaccine was found to be a newly described primary cause of BCS.https://digitalcommons.unmc.edu/surp2021/1046/thumbnail.jp